CLINICAL AND LABORATORY PROFILE OF PATIENTS WITH IDIOPATHIC CD4 LYMPHOCYTOPENIA- A RARE CLINICAL ENTITY

Abstract

Vijayashree Thyagaraj 1 , Karthik Ashok

BACKGROUND Since 1989, several investigators have reported unusual cases of severe opportunistic infections associated with CD4 lymphocytopenia in the absence of human immunodeficiency virus infection. The cause of this condition is unknown. The Centres for Disease Control and Prevention (CDC) defines Idiopathic CD4 T Lymphocytopenia (ICL) as a clinical condition in which patients with depressed numbers of circulating CD4+ T-cell lymphocytes (<300 cells/μL or <20% of total T cells) at a minimum of two separate time points at least 6 weeks apart, have no laboratory evidence of infection with human HIV-1 or HIV-2, or any defined immunodeficiency or therapy associated with depressed levels of CD4 T cells. The aim of the study is to analyse the clinical profile, opportunistic infections, laboratory parameters and outcome in terms of survival of patients diagnosed with ICL. MATERIALS AND METHODS Eight HIV negative patients who presented with opportunistic infections and who were diagnosed with ICL from 2007 to 2015 were included in the study. A detailed history was taken; physical examination was performed and the nature of illness with which they presented was documented. Then, CD4 and CD8 counts were done and CD4 count was repeated after a 6-week interval. The patients were followed up until discharge or death. RESULTS The mean age was 37.50±9.55 years. There were six males (75%) and two females (25%). Fever was a presenting symptom among six (75%) of them. Two were diagnosed to have cutaneous cryptococcosis (25%), two with invasive aspergillosis (25%) and four with tuberculosis (50%). Absolute lymphocyte count was less than 1200 in seven patients (87.5%), which roughly correlates with a CD4 count of less than 200 cells/μL, among PLWHIV. The mean CD4 count was 183.63±63.74 cells/μL during the first measurement and 214.43±103.98 cells/μL during the second one. Two patients died (37.5%). None of the patients were recorded to have any form of malignancy. CONCLUSION ICL mostly affects young individuals with unusual opportunistic infections, the most common being tuberculosis and cryptococcosis. There was no worsening of the CD4 count with time and death in our patients was not directly related to the condition.

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